Clinical Manifestation and Survival of Patients with I Diopathic Bilateral
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ORIGINAL ARTICLE Clinical Manifestation and Survival of Patients with Mizuhiro Arima, TatsujiI diopathicKanoh, Shinya BilateralOkazaki, YoshitakaAtrialIwama,DilatationAkira Yamasaki and Sigeru Matsuda Westudied the histories of eight patients who lacked clear evidence of cardiac abnormalities other than marked bilateral atrial dilatation and atrial fibrillation, which have rarely been dis- cussed in the literature. From the time of their first visit to our hospital, the patients' chest radio- graphs and electrocardiograms showed markedly enlarged cardiac silhouettes and atrial fibrilla- tion, respectively. Each patient's echocardiogram showed a marked bilateral atrial dilatation with almost normal wall motion of both ventricles. In one patient, inflammatory change was demonstrated by cardiac catheterization and endomyocardial biopsy from the right ventricle. Seven of our eight cases were elderly women.Over a long period after the diagnosis of cardiome- galy or arrhythmia, diuretics or digitalis offered good results in the treatment of edema and congestion in these patients. In view of the clinical courses included in the present study, we conclude that this disorder has a good prognosis. (Internal Medicine 38: 112-118, 1999) Key words: cardiomegaly, atrial fibrillation, elder women,good prognosis Introduction echocardiography. The severity of mitral and tricuspid regur- gitation was globally assessed by dividing into three equal parts Idiopathic enlargement of the right atrium was discussed by the distance from the valve orifice. The regurgitant jet was de- Bailey in 1955(1). This disorder may be an unusual congenital tected on color Doppler recording in the four-chamber view malformation. A review of the international literature disclosed and classified into one of the three regions (-: none, +: mild, that although several cases have been discussed since Bailey's ++:moderate, +++: severe). Definite pulmonary hypertension report (2-17), idiopathic bilateral atrial dilatation is not well was defined as more than 25 mmHg, which was calculated by known (18-20). We discuss eight patients with bilateral atrial Doppler echocardiography (-: none, +: mild). Patients with le- dilatation who presented with no other cardiac abnormalities sions known to be secondary to atrial enlargement were ex- exceptfor atrial fibrillation. cluded. We investigated the clinical features and treatment course of each patient. Results of chest radiographs, electro- Methods cardiograms, and two-dimensional and color Doppler echocardiograms, when available, were examined. Four patients We studied the histories of eight patients, one man and seven had undergone magnetic resonance imaging. Wewerenot able women, treated for bilateral atrial dilatation within the past 13 to perform all of the tests on the more elderly patients. years at our hospital. The cardiothoracic ratio exceeded 65% Transesophageal echocardiography had been performed on in the patients studied. We used two-dimensional and color three patients. Cardiac catheterization and endomyocardial bi- Doppler echocardiographic findings to determine the follow- opsy from the right ventricle were performed on one patient. ing: 1) a left atrial short dimension of more than 55mm in the left parasternal window; 2) a right atrial short dimension of more than 55mm in the four-chamber view; 3) no disturbance Results in left ventricular wall motion and no structural abnormalities atient characteristics and clinical features (Table 1) of the tricuspid, mitral, aortic, and pulmonary valve complexes; The patient group consisted of one manand seven women 4) no severe mitral regurgitation and/or aortic regurgitation, with a mean age of 70 years (range, 47 to 87 years). Dyspnea and no abnormal shunt flow confirmed by color Doppler wasP the chief complaint of four patients, and generalized edema From the Division of Cardiology, Department of Internal Medicine, Juntendo Urayasu Hospital, Juntendo University School of Medicine, Chiba Received for publication February 25, 1998; Accepted for publication November 9, 1998 eprint requests should be addressed to Dr. Mizuhiro Arima, the Division of Cardiology, Department of Internal Medicine, Juntendo Urayasu Hospital, R Juntendo University School of Medicine, 2-1-1 Tomioka, Urayasu, Chiba 279-0021 112 InternalMedicineVol. 38, No. 2 (February 1999) Idiopathic Bilateral Atrial Dilatation Table 1. Patient Characteristics and Clinical Features C a s e N o . S e x A g e (y e a rs) c . c . C T R ( % ) E C G D ur a ti o n ( ye a rs ) C H F 1 F 8 0 D y sp n e a A F C R B B B L A D 3 7 + 2 F 4 7 E d e m a 6 5 A F I R B B B 1 6 3 F 8 7 E d e m a 7 0 A F L o w V o l t a g e 3 2 + 4 F 8 3 P alp itatio n 8 5 A F 4 0 D e a t h 4- 5 F 7 0 D y sp n e a 6 8 A F L V H 2 8 + 6 F 6 8 E d e m a 7 2 A F 3 8 + 7 F 7 4 D y sp n e a 7 8 A F I R B B B 3 4 8 M 5 2 D y sp n e a 6 8 A F l l C. C : chief complaint, CTR: cardiothoracic ratio, ECG: electrocardiogram, Duration (year): duration after diag- nosis of cardiomegaly or arrhythmia, Case 4 died of cerebral infarction. CHF: congestive heart failure, -: none, +.CHF (Class III or Class IV on the NYHAscale), F: female, M: male, AF: atrial fibrillation, CRBBB:complete right bundle branch block, IRBBB: incomplete right bundle branch block, LAD: left axis deviation, Low Volt- age: low voltage in limb lead, LVH:left ventricular hypertrophy with inversion of T wave and high voltage in left precordial lead. was the chief complaint of three patients. Chest radiographs effective in these cases. One patient died of cerebral infarction showed markedly enlarged cardiac silhouettes from the time at the age of 83. Nopatient died as the result of congestive of the patients' first visit to our hospital. The range of the most heart failure. recent cardiothoracic ratios was from 65%to 88%, with a mean of 143%. The electrocardiograms showed atrial fibrillation in Changes in the cardiothoracic ratio (Fig. 1) all patients, incomplete right bundle branch block in two pa- Changes in the cardiothoracic ratio in the compensated state tients, complete right bundle branch block and left axis devia- of congestive heart failure are shown in Fig. 1. The cardio- tion in one patient, and left ventricular hypertrophy in one pa- thoracic ratio increased gradually. The range of cardiothoracic tient. Three patients were treated with digitalis because of atrial ratios from the first visit to our hospital was from 57%to 80%, fibrillation with rapid ventricular response. The meanduration with a meanof 68.5%, and the range from the most recent visit after diagnosis of cardiomegaly or arrhythmia was 29.5 years was from 65%to 88%, with a mean of74.3%. (range, 1 1 to 40 years). Five patients were categorized as Class III or Class IV on the NewYorkHeartAssociation scale (NYHA Findings of two-dimensional and color Doppler echocardio- scale) during their clinical course. Although six patients were grams (Table 2) admitted to our hospital because of worsening edemaor con- In each case, the echocardiogram showeda markedbilat- gestive heart failure, treatment with diuretics or digitalis was eral atrial dilatation with almost normal wall motion of both CTR (%) 100_ -#- Case 1 -O- Case 2 90- ^^» -å²- Case3 -+^^å80- a----'* /V" ^^ ^ å -T- Case5 + 70_ V V ^-^^f -V-Case6 i__ * ^g^PP^ ---Case7 V--~~~J*^^^ 60- CT -G- Case8 50- 40- 30- '85 '86 '87 '88 '89 '90 '91 '92 '93 '94 '95 '96 '97 (Year) Figure 1. Changes in the cardiothoracic ratio. InternalMedicine Vol. 38, No. 2 (February 1999) 113 Arima et al Table 2. Findings of Two-Dimensional and Color Doppler Echocardiograms L V D d L V D s E F IV S L V P W L A D R A D T R M R P H Case No. (m m ) (m m ) (m m ) (m m ) (m m ) (m m ) 1 4 8 3 0 0 .7 6 1 2 1 3 6 9 8 0 + + + + + 2 4 0 2 6 0 .7 2 1 0 l l 7 0 7 6 + + 3 4 9 3 2 0 .7 2 1 0 1 0 6 5 6 8 + 4 4 2 3 0 0 .6 4 l l 1 0 7 0 6 9 + + + + 5 4 2 2 8 0 .7 0 1 2 1 2 5 5 5 8 + + + 6 3 9 2 7 0 .6 6 l l l l 6 0 6 0 + + 7 5 0 3 0 0 .7 8 1 0 1 0 6 1 6 4 + + 8 4 1 2 5 0 .7 7 1 0 1 0 6 5 6 0 + + LVDd: left ventricular diastolic dimension, LVDs: left ventricular systolic dimension, EF: ejection fraction, IVS: interventricular septal thickness, LVPW: left ventricular posterior wall thickness, LAD left atrial dimen- sion, RAD: right atrial dimension, TR: tricuspid regurgitation, MR: mitral regurgitation, PH: pulmonaly hyper- tension. ventricles. The left ventricular end diastolic dimension was gitation. Upon treatment with diuretics, the edema and con- 43.914.1 mm, and the ejection fraction was 0.72±0.05. Wall gestion disappeared, but the cardiomegaly improved only thickness of the left ventricle was within the normal limits in slightly. Follow-up chest radiographs are shown in Fig. 2. The all but one patient. The average dimension of the left atrium patient's cardiothoracic ratio increased gradually. Magnetic was 64.4±5.0 mm and that of the right atrium was 66.9±7.4 resonance imaging revealed a bilateral atrial dilatation in the mm.